cells and tissues 2 Flashcards
what are the layers of the basement membrane?
lamina lucida (top) lamina densa (bottom)
what is laminin?
protein of the basal lamina
explain the cause of Marfin’s syndrome
connective tissue disorder
elastin normally polymerises into fibrils and fibrillin coats it which limits how far it can stretch
in marfan’s, the fibrillin is misfolded so allows body parts to stretch abnormally - elastin cant recoil
what type of collagen is the basement membrane?
collagen type 4
name a disease caused by the mutation of laminin
junctional epidermolysis bullosa
explain the cause of junctional epidermolysis bullosa and how it presents
laminin is mutated
skin v fragile
blisters form in the lamina lucida
what are the types of epithelial junctions?
tight gap adherens hemidesmosomes desmosomes
what do tight junctions do?
prevent passage of molecules across the epithelium
what do adherens do?
tether adjacent cells together
what do desmosomes do?
resist mechanical stress
what do gap junctions do?
allow small passage of molecules through
what do hemidesmosomes do?
anchor epithelium to basal lamina
where are lots of desmosomes found?
in the heart
what do mutations of demosomes generally cause?
diseases of the skin and heart (impaired cardiac function)
what causes pemphigus vulgaris?
immune system attacks desmosomal proteins using autoantibodies causing epidermal blisters
what causes Staph. scalded skin syndrome?
when bacterial proteins are aimed at the desmosomal cadherins causing epidermal blisters
what causes Naxos disease and how is it presented?
genetic disease - mutation of desmoplakin
causes skin blisters, cardiomyopathy and wooly/curly hair
what is the function of non-motile cilia?
sensory antennae on all cells
what is the function of motile cilia?
to move things along